Xu Jiaying, Wei Min, Miao Qi, Zhu Bin, Yu Chunhua, Huang Yuguang
Department of Anesthesiology Department of Cardiac Surgery, Chinese Academy of Medical Sciences/Peking Union Medical College Hospital Department of Anesthesiology, Peking University International Hospital, Beijing, P.R. China.
Medicine (Baltimore). 2017 Jul;96(29):e7522. doi: 10.1097/MD.0000000000007522.
Intracardiac leiomyomatosis (ICLM) is a rare condition in which the benign tumor extends into the right heart chambers through inferior vena cava. The best surgical approach still remains unclear.We present a retrospective cohort of 36 patients diagnosed with ICLM in Peking Union Medical College Hospital between 2002 and 2016.The mean patient age was 44.5 (range 25-55) years. The clinical manifestations were various, including shortness of breath, chest tightness, edema of the lower extremities, palpitations, syncope, etc. Cardiac function of 30 patients (80%) remained mildly influenced, classified as New York Heart Association (NYHA) I-II. After careful preoperative evaluation, 19 patients underwent 1-stage operation while the other 17 patients underwent 2-stage operations. The original surgical plans were changed in 5 patients (14%) due to intraoperative transesophageal echocardiography (TEE) monitoring, with the tumor directly extracted through abdominal approach or right atrium without cardiopulmonary bypass and/or deep hypothermic circulatory arrest. Complete resection was achieved in 32 patients (89%). Despite increased volume of blood loss (P < .05), patients undergoing 1-stage operation had significantly shorter operation time, anesthesia time as well as hospital length of stay (P < .05), compared with 2-stage operations. The postoperative complication rates were not different between the 2 groups (P = .684). During mean follow-up time of 36.1 months, recurrence occurred in 7 patients (23%) but all are survived.Precise and full-scale preoperative evaluation of both the tumor anatomy and the patient's tolerability to the surgery should be performed. TEE plays a crucial role in guidance of surgical decision making, and 1-stage extraction of tumor through either abdominal approach or right atrium may be possible.
心脏内平滑肌瘤病（ICLM）是一种罕见疾病，其中良性肿瘤通过下腔静脉延伸至右心腔。最佳手术方法仍不明确。我们回顾性分析了2002年至2016年在北京协和医院诊断为ICLM的36例患者。患者平均年龄为44.5岁（范围25 - 55岁）。临床表现多样，包括气短、胸闷、下肢水肿、心悸、晕厥等。30例患者（80%）的心功能仍受到轻度影响，纽约心脏协会（NYHA）心功能分级为I - II级。经过仔细的术前评估，19例患者接受了一期手术，另外17例患者接受了二期手术。5例患者（14%）因术中经食管超声心动图（TEE）监测而改变了原手术计划，肿瘤通过腹部途径或右心房直接摘除，无需体外循环和/或深低温停循环。32例患者（89%）实现了完全切除。与二期手术相比，尽管一期手术患者失血量增加（P<0.05），但其手术时间、麻醉时间以及住院时间显著缩短（P<0.05）。两组术后并发症发生率无差异（P = 0.684）。在平均36.1个月的随访时间内，7例患者（23%）复发，但均存活。应进行精确且全面的术前评估，包括肿瘤解剖结构和患者对手术的耐受性。TEE在指导手术决策中起关键作用，通过腹部途径或右心房一期摘除肿瘤是可能的。
